Search Results (7)

Right atrial isomerism (RAI) is a complex entity with varying diagnostic and treatment outcomes due to its rarity. Treatment options range from palliative to corrective surgeries, resulting in heterogeneous outcomes. The aim of this study was to analyze the results obtained after cardiac surgery in patients with RAI. A retrospective study was conducted, including patients diagnosed with RAI who underwent cardiac surgery. Their follow-up was from 1 January 2010 to 31 March 2020. Demographic characteristics and perioperative conditions were described. Thirty-eight patients were included, the median age was 4 years (IQR 2–9.2) and 57.9% were men. The main diagnoses were atrioventricular canal (63.2%) and pulmonary stenosis (55.3%). The most common surgical procedures were modified Blalock–Taussig shunt (65.8%) and total cavopulmonary connection with an extracardiac conduit fenestrated without cardiopulmonary bypass (15.9%). We did not find any factors associated with negative outcomes in these patients. The overall survival was 86.8%, with a better outcome in those who did not require reintubation (log rank, p < 0.01). The survival of RAI was similar to other centers. Individuals with RAI should be evaluated rigorously to determine an adequate repair strategy, considering high morbidity and mortality. Full article

Clinical presentation, course, and treatment for patients with isolated right ventricular (RV) hypoplasia (IRVH) depends on the degree of hypoplasia that is present—this is a spectrum from spontaneous maturation to Fontan circulation over time. An 8-month-old infant presented with IRVH; in the patient, a modified Blalock–Taussig (MBTS) shunt was closed percutaneously after spontaneous RV function recovery. A female newborn was diagnosed with differential cyanosis at birth. The echocardiography showed a hypertrophic RV with a small cavity, a right–left shunt on the atrial septal defect, an almost closed ductus arteriosus (DA), and a small tricuspid valve ring (Z-score-2) with mild regurgitation (pressure gradient 30 mmHg). On the 4th day of life, the patient showed deepened cyanosis and hyperlactatemia was registered. The echocardiography examination revealed a closed DA. Right ventriculography performed on the 5th day of life evidenced the presence of a small hypertrabeculated RV. The pressure in the RV increased. A right-side MBTS was created on the 6th day of life. Further echocardiographic findings indicated a gradual development of the RV and a decrease in RV pressure. MBTS occlusion was performed when the patient was 8 months old. Vital parameters were monitored invasively and noninvasively after the balloon occlusion of MBTS. Percutaneous MBTS occlusion was successfully performed using an Amplatzer vascular plug 2 (AVP2). During the follow-up period, the patient was found to have maintained a normal percutaneous oxyhaemoglobin blood saturation. Full article

Jacobsen syndrome (JS) is a rare syndrome caused by a deletion of chromosome 11q. We report a patient with JS and hypoplastic left heart syndrome (HLHS) who required cardiac transplantation. She had many of the recognized morphological features in addition to immunological (lymphopenia) and hematological (thrombocytopenia) issues. The patient underwent a Norwood procedure with a modified Blalock–Taussig shunt (MBTS) and subsequently a Glenn procedure at six months of age. She developed desaturation, with severe tricuspid regurgitation and right ventricular dysfunction, and underwent heart transplantation at 7 months of age. After the transplant, she was hospitalized several times for severe infections. The diagnosis of Jacobsen syndrome came 2 months after transplant. Now, 5 years post-transplant, she is in relatively good health—her heart is functioning normally, her hospitalization rate is getting lower, and her immunological profile is stable. Full article

The Modified Blalock Taussig Shunt (MBTS) is one of the most common palliative operations in case of cyanotic heart diseases. Thus far, the decision on the position, size, and geometry of the implant relies on clinicians’ experience. In this paper, a Medical Digital Twin pipeline based on reduced order modeling is presented for fast and interactive evaluation of the hemodynamic parameters of MBTS. An infant case affected by complete pulmonary atresia was selected for this study. A three-dimensional digital model of the infant’s MBTS morphology was generated. A wide spectrum of MBTS geometries was explored by introducing twelve Radial Basis Function mesh modifiers. The combination of these modifiers allowed for analysis of various MBTS shapes. The final results proved the potential of the proposed approach for the investigation of significant hemodynamic features such as velocity, pressure, and wall shear stress as a function of the shunt’s morphology in real-time. In particular, it was demonstrated that the modifications of the MBTS morphology had a profound effect on the hemodynamic indices. The adoption of reduced models turned out to be a promising path to follow for MBTS numerical evaluation, with the potential to support patient-specific preoperative planning. Full article

Cardiovascular surgery requires the use of state-of-the-art artificial materials. For example, microporous polytetrafluoroethylene grafts manufactured by Gore-Tex^® are used for the treatment of cyanotic heart defects (i.e., modified Blalock–Taussig shunt). Significant mortality during this palliative operation has led surgeons to adopt mathematical models to eliminate complications by performing fluid–solid interaction (FSI) simulations. To proceed with FSI modeling, it is necessary to know either the mechanical properties of the aorta and graft or the rheological properties of blood. The properties of the aorta and blood can be found in the literature, but there are no data about the mechanical properties of Gore-Tex^® grafts. Experimental studies were carried out on the mechanical properties vascular grafts adopted for modified pediatric Blalock–Taussig shunts. Parameters of two models (the five-parameter Mooney–Rivlin model and the three-parameter Yeoh model) were determined by uniaxial experimental curve fitting. The obtained data were used for patient-specific FSI modeling of local blood flow in the “aorta-modified Blalock–Taussig shunt–pulmonary artery” system in three different shunt locations: central, right, and left. The anisotropic model of the aortic material showed higher stress values at the peak moment of systole, which may be a key factor determining the strength characteristics of the aorta and pulmonary artery. Additionally, this mechanical parameter is important when installing a central shunt, since it is in the area of the central anastomosis that an increase in stress on the aortic wall is observed. According to computations, the anisotropic model shows smaller values for the displacements of both the aorta and the shunt, which in turn may affect the success of preoperative predictions. Thus, it can be concluded that the anisotropic properties of the aorta play an important role in preoperative modeling. Full article

The current treatment practice for Tetralogy of Fallot (TOF) is to undertake complete surgical repair between 6–9 months of age with excellent immediate and long-term results. In patients with increased cyanosis or frequent cyanotic spells, younger age of complete repair as early as 3 months is usually acceptable. Although neonatal TOF repair is reported with good immediate survival from a few centres, post-operative morbidity is significant, and the hospital stay is prolonged. Hence, in neonates and small term and preterm infants with severe cyanosis, palliative procedures such as modified Blalock-Taussig (BT) shunt, ductal stenting, right ventricular outflow tract (RVOT) stenting and balloon pulmonary valvotomy (BPV) have been reported. With the development of low-profile stents, an increasing clinician experience and more predictable outcome with both duct and RVOT stenting, these procedures have gained popularity as the preferred palliations for TOF at most centres. We reviewed the literature to analyse whether BPV for TOF palliation, a technique first reported three decades ago, still has a role in the present era of paediatric cardiac care. In this review, we have concluded that BPV has very specific indications in patients with TOF, with predominantly valvar pulmonary stenosis, and may be a preferred option over other palliative strategies in hypoxemic preterm or low-birth weight infants. Full article

In this review management of the most common cyanotic congenital heart defects (CHDs) was discussed; the management of acyanotic CHD was reviewed in Part I of this series. While the need for intervention in acyanotic CHD is by and large determined by the severity of the lesion, most cyanotic CHDs require intervention, mostly by surgery. Different types of tetralogy of Fallot require different types of total surgical corrective procedures, and some may require initial palliation, mainly by modified Blalock–Taussig shunts. Babies with transposition of the great arteries with an intact ventricular septum as well as those with ventricular septal defects (VSD) need an arterial switch (Jatene) procedure while those with both VSD and pulmonary stenosis should be addressed by Rastelli procedure. These procedures may need to be preceded by prostaglandin infusion and/or balloon atrial septostomy in some babies. Infants with tricuspid atresia require initial palliation either with a modified Blalock–Taussig shunt or banding of the pulmonary artery and subsequent staged Fontan (bidirectional Glenn and fenestrated Fontan with extra-cardiac conduit). Neonates with total anomalous pulmonary venous connection are managed by anastomosis of the common pulmonary vein with the left atrium either electively in non-obstructed types or as an emergency procedure in the obstructed types. Babies with truncus arteriosus are treated by surgical closure of VSD along with right ventricle to pulmonary artery conduit. The other defects, namely, hypoplastic left heart syndrome, pulmonary atresia with intact ventricular septum, double-outlet right ventricle, double-inlet left ventricle and univentricular hearts largely require multistage surgical correction. The currently existing medical, trans-catheter and surgical techniques to manage cyanotic CHD are safe and effective and can be performed at a relatively low risk. Full article